Chemical Name: N-Butyldeoxynojirimycin HCl. Alternate Names: NB-DNJ
alpha-glucosidase inhibitor
Molecular Weight:
255.74
Purity:
98% by TLC, NMR (Conforms)
Form:
White solid
CAS Number:
[210110-90-0]
Formula:
C10H21NO4 HCl
Application Notes:
Orally active ceramide-specific glycosyltransferase and alpha-glucosidase I and II inhibitor.1 Rescues trafficking-deficient F508del-CFTR in human airway epithelial cells via inhibition of ER alpha-glucosidases I and II.2 Pharmacological chaperone for glucocerebrosidase degradation.3 Clinically useful agent for Gaucher disease type 1.4 Stabilizes neurological disorders in Niemann-Pick disease type C.5
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